Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Various abnormal growths appear on planarians, Dugesia dorotocephala, during and after exposure to polychlorinated biphenyls (PCBs) 28, 110, and 126; Aroclor 1254; cadmium sulfate; and L-buthionine-(R,S)-sulfoximine (BSO). Daily observations under magnification were used to describe the location, development, and morphology of three different types of tumor-like growths ("tumors"). "Post-head tumors" were found to be highly invasive, progressive, and lethal to the animal depending on concentrations and combinations of the compounds used. Survivors from post-head tumors exhibited aberrant morphogenesis, but developmental abnormalities were eventually shed. Post-head tumors occurred within 2 weeks of initial exposure, while "round tail tip tumors" appeared after 2-3 weeks. The rate of progression and invasiveness was greater for the round tail tip tumors. "Pigmented rose thorn tail tumors" occurred in low incidence (4-20%) and appeared to be harmless and noninvasive, requiring months to develop from the first appearance of pigmentation. The aggressive, proliferative, and invasive characteristics of post-head and round tail tip tumors are analogous to those of malignant tumors, while pigmented rose thorn tumors were benign. High dose of cadmium alone were sufficient to initiate the post-head and round tail tip tumors. PCBs potentiated the tumorigenicity of low cadmium doses and enhanced the very low spontaneous incidence of pigmented rose thorn tumors. PCBs also impaired motor activity, causing the graceful gliding locomotion to be replaced by a twisting serpentine movement accompanied by muscular dystrophy. In addition, high (50 micrograms) doses of PCB 110 depressed activity, while lower (5 micrograms) doses and 50 micrograms Aroclor 1254 induced restlessness and enhanced locomotion. These data provide the basis for quality assurance.
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PMID:A scientific basis for proposed quality assurance of a new screening method for tumor-like growths in the planarian, Dugesia dorotocephala. 134 77

Patients with neuromuscular disease may suffer from nocturnal respiratory failure despite normal daytime respiratory function. The physiological reduction in muscle tone during sleep may be life-threatening in a patient with impaired muscle strength. Nocturnal respiratory failure may occur in patients with the postpolio syndrome, amyotrophic lateral sclerosis, myasthenia gravis, myotonic dystrophy, and muscular dystrophy. Diagnosis of obstructive, central and mixed apneas, hypopneas, and hypoventilation is best made using polysomnography. Therapeutic options include noninvasive ventilation such as continuous positive airway pressure, bilevel positive airway pressure, intermittent positive pressure ventilation and, rarely, tracheostomy, oxygen, or protriptyline. Evaluation by a sleep specialist should be initiated in any neuromuscular patient with nocturnal symptoms such as air hunger, intermittent snoring or breathing, orthopnea, cyanosis, restlessness, and insomnia. Daytime symptoms may include morning drowsiness, headaches and excessive daytime sleepiness. Polycythemia, hypertension, and signs of heart failure may also be seen. Effective treatment is available, and may improve the quality of life, and possibly increase survival.
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PMID:Nocturnal respiratory failure as an indication of noninvasive ventilation in the patient with neuromuscular disease. 967 Mar 10

The effects of different types of cell carriers, strategies for cell transfer on carriers, and of several fusion inhibitors on the growth kinetics of primary human myoblasts culture were studied in order to develop a bioprocess suitable for the treatment of Duchenne muscular dystrophy based on the transplantation of unfused cells. Our results indicate that myoblast production is larger on Cytodex 1 and 3 than on polypropylene or polyester fabrics and on a commercial porous macrocarrier. Myoblast growth conditions with Cytodex 1 were further investigated to establish the bioprocess operating conditions. It was found that microcarrier density of 3 g DW l(-1), inoculum density of 2x10(5) cells ml(-1), and continuous agitation speed of 30-rpm result in final myoblast production comparable to static cultures. However, for all the culture conditions used, myoblasts growth kinetics exhibited a lag phase that lasted a minimum of 1 week prior to growth, the end of the lag phase correlating with the appearance of microcarrier aggregates. Based on this observation, we propose that aggregation promotes cell growth by offering a network of very large inter-particular pores that protect cells from mechanical stress. We took advantage of the presence of these aggregates for the scale-up of the culture process. Indeed, using myoblast-loaded microcarrier-aggregates instead of myoblast suspension to inoculate a fresh suspension of microcarriers significantly reduced the duration of the lag phase and allowed the scale-up of the bioprocess at the 500-ml scale. In order to ensure the production of unfused myoblasts, the efficiency of five different fusion inhibitors was investigated. Only calpeptin (9.1 microg ml(-1)) significantly inhibited the fusion of the myoblasts, while TGFbeta (50 ng ml(-1)) and LPA (10 microg ml(-1)) increased myoblasts growth but did not affect fusion, sphingosine (30 microg ml(-1)) induced a 50% death and NMMA (25 microg ml(-1)) had no effect on either growth or fusion. Finally, transplantation trials on severe combined immunodeficient mice showed that microcarrier-cultured human myoblasts grown using the optimized bioprocess resulted in grafts as successful as myoblasts grown in static cultures. The bioprocess, therefore, prove to be suitable for the large-scale production of myoblasts required for muscular dystrophy treatment.
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PMID:Scale-up of a myoblast culture process. 1152 63